Twin Twin Transfusion Syndrome: What are the ... - March of Dimes

Twin Twin Transfusion 

Syndrome: What are the 

Outcomes? 

David F. Lewis MD 

Professor and Vice Chair 

University of Cincinnati 

Fetal Care Center of Cincinnati

TWIN-TWIN TRANSFUSION SYNDROME 

Unsolved problem 

• Most common complication in MC 

twins 

• 44-35% 35% of all MC gestations in US 

• 0.1-0.9 per 1000 births 

• 17% of all perinatal p mortality yin 

twins 

• Mortality of 80-100% if untreated 

• Mortality of 15-63% 15 63% even with 

treatment

Fetal Care Center of Cincinnati 

TWIN TROUBLES 

1118 sets of twins 

27 Conjoined twins 

198 Anomalous Co-twins 

67 TRAP Sequence q 

826 TTTS 

Twin-Twin Transfusion Syndrome

Twin-Twin Transfusion Syndrome 

Diagnostic Criteria 

• Neonatal criteria 

− Discordant cord blood Hgb > 5 g/dl 

− Discordant birth weight > 20% 

• Non-TTTS Non TTTS meeting criteria 

− 19/130 had Hgb > 5 g/dl 

− Growth restriction without TTTS 

− Fetal acidosis acidosis, hypoxemia 

− Polycythemia 

• Discordant Hgb rare in TTTS 

• Discordant weight eight common in TTTS 

− ? threshold of >20%

Quintero Staging of TTTS 

Stage I: Discordant amniotic fluid: DVP >8 cm & < 2 cm 

Stage II: Absent bladder in the donor 

Stage III: Doppler velocimetry changes in UA, UV, DV 

Stage IV: Hydrops 

St Stage V: V DDeath th of f one ffetus t


Diagnostic g Evaluation 

•Echocardiogram 

•Fetal MRI 

•Ultrasound 

• Monochorionic gestation 

• Oligohydramnios DVP < 2cm 

• Polyhydramnios DVP > 8cm 

• Doppler velocimetry changes 

• Donor 

• AEDF in UA 

• Complete absence of DF in 

UA 

• RRecipient i i t 

• Pulsatile UV 

• AEDF in UA 

• DV: decreased, absent, 

reversed a wave 

• Growth discordance

TTTS DIAGNOSIS OF EXCLUSION 

•15% cases not TTTS 

•15% cases not TTTS 

•TTTS mimicked by 

• Placental insufficiency 

• Dichorionic gestation 

• Discordant anomaly 

• Discordant viral 

infection

Hydrops/ 

Ascites 

Fetal MRI Findings in TTTS 

Recipient p Donor 

Cases 

DCVS 

Cases 

DCVS 

15% 6% 

B Brain i Bleed/ Bl d/ 15% 3% 

Ischemia 

Demise 4% 6%


TTTS Cardiomyopathy 

• Cardiac decompensation 

• More afterload pathology p gy 

• Not volume stress 

• Isolated tricuspid regurgitation 

• RV hypertension yp Systolic y P> 80 

mmHg 

• Pulmonary outflow tract 

obstruction 

• Pulmonary insufficiency 

• Pulmonary atresia/intact 

ventricular septum p

EEchocardiographic h di hi Fi Findings di iin TTTS 

•Myocardial Myocardial hypertrophy RV > 

LV 

•Decreased compliance RV > LV 

−Monophasic inflow 

•AV AV valve l iincompetence t TV > 

MV 

•Poor compliance/ AV valve 

incompetence reflected in 

•DV abnormalities 

−Decrease in a wave 

−Absence of a wave 

−Reversal of a wave 

•Acquired congenital heart 

disease 

−Pulmonic valve stenosis, insufficiency, 

atresia 

−Not reversible with treatment of TTTS

Treatments for Twin-Twin 

Transfusion Transfusion Syndrome 

Syndrome 

•Maternal Digoxin 

•Maternal Maternal Indocin 

•Amnioreduction 

•Selective fetocide 

•Hysterotomy Hysterotomy for cord ligation 

•Blood letting of recipient 

•Septostomy 

•Microseptostomy 

Microseptostomy 

•Non-selective Laser 

photocoagulation 

•Selective Laser photocoagulation 

•Fetoscopic cord ligation 

•Fetoscopic RFA 

•Fetoscopic cord coagulation


Amniocentesis 

• Standard therapy in US 

• IInitially iti ll treated t t dmaternal t l 

symptoms 

• Survival of 50% 

• Moise Semin Perinatol 1993 

• Survival in aggressive 

amnioreduction 

• 37% to 83% 

• Minimal maternal or fetal risk 

• Incidence of neurologic ne rologic 

abnormalites 

• 18 to 26 % of survivors


• International Registry on 

TTTS Treated by 

Amnioreduction 

• 223 pregnancies < 28 weeks 

• Average g number of 

amnioreductions 2 

• Complications-mostly fetal 

− PPROM 6.2% 6 2% 

− Labor 3.1% 

− Fetal demise 3.4% 

− Placental abruption 1.3% 

− Chorioamnionitis 0.9% 

− Miscarriage 0.4% 

• Mari a et al a Am J Obstet Gynecol Gy eco 

185:708-715,2001 

Amniocentesis

Twin-Twin Twin Twin Transfusion Transfusion Syndrome 

IInternational t ti l Amnioreduction A i d ti Registry R i t 

Mari et al 

• Survival to birth 78% 

• Survival to 4 weeks of age 60%

SEPTOSTOMY 

• Single amnioreduction paradox 

• Inadvertent septostomy 

• Saade et al Am J Obstet Gynecol 172: 429, 1995 

• Saade et al Fetal Diagn Ther 13:868-93 

− 12 patient ti t 

− Survival 83% at GA of 31 wks 

• Hubinot et al Euro J Obstet Gynecol Reprod Biol 92: 14, 2000 

− 7patients 7 patients 

− Survival 57% 

• Johnson et al Am J Obstet Gynecol 185: 1044, 2001 

− Single institution: 7 cases AR; 7 cases septostomy 

− Survival for AR 64%: 

− Survival for septostomy 71% 

• Moise et prospective trial comparing AR to septostomy 

− 65% survival with AR and with septostomy 

Septostomy

TEMPORIZING MICROSEPTOSTOMY 

•Eliminates repeated amnioreductions 

•Decreased risk of chorioamniotic 

separation 

•Resuscitation of “stuck” twin 

•Temporizing measure in TTTS 

•Lose oligo/poly as sign 

•TTTS’s hemodynamic derangement 

− Serial ultrasounds 

− Serial echocardiograms 

•Microseptostomies 

Mi t t i 

− 1/3 stabilized 

− 2/3 progressed hemodynamically 

Microseptostomy

DETECTING PROGRESSION OF TTTS 

•ULTRASOUND 

FINDINGS 

Ductus Venosus 

− Serial examinations 

− 

− 

Hydrops y p 

Progressive growth 

discordance-2 wk 

Flow Reversal 

•DOPPLER 

VELOCIMETRY 

− Absent or reversed 

diastolic flow in UA 

Umbilical Artery 

− Reversal of flow in 

Ductus Venosus Absent Diastolic flow

FETAL ECHOCARDIOGRAPHIC 

SIGNS OF PROGRESSION OF TTTS 

•Development or 

worsening TR 

•Development or 

worsening MR 

•Development of PI/PS 

•Worsening ventricular 

hypertrophy 

•Reduced ventricular 

fractional shortening 

•Tei myocardial 

performance index 

TR jet


Non-Selective Non Selective Fetoscopic 

Laser Photocoagulation 

• 1990 DeLia 

− Ob Obstet GGynecol l 75 75:1046, 1046 1990 

• 1995 DeLia 

− Am J Obstet Gynecol 

172:1202,1995 

− 53% survival 

− 96% “developing 

normally” 

• 1995 995 Ville V e 

− N Engl J Med 332;224, 

1995 

− 53% survival 

− Survivors “developing developing 

normally” 

Inter-twin Membrane


Non-Selective or Semi-Selective 

Fetoscopic Laser Photocoagulation 

Fetal Survival At least 1 Survivor 

DeLia ‘95 52.8% 69.2% 

DeLia ‘99 69% 82% 

Ville ‘98 54.5% 73.5% 

Senat ‘04 57% 76% 

Inter-twin Membrane


Sequential Selective Fetoscopic Laser 

Photocoagulation 

Quintero et al J Matern Fetal Neonatal Med 2007 

• Sequential selective laser photocoagulation of communicating 

vessels in twin-twin transfusion syndrome Quintero et al J 

Matern Fetal Neonatal Med 10: 763-768, 2007 

• Donor AV first, then Recepient AV then A-A then VV 

• Favorable results 

• Likely due to detailed mapping 

• Shorter duration of laser use 

• CComparable bl results lt to t FCC


SelectiveFetoscopic Laser Photocoagulation 

•1998 Quintero et al 

− Obstet Gynecol Surv 

53:597 53:597,1998 1998 

•1999 Hecher et al 

− Am J Obstet Gynecol 180: 

717, 1999 

• 73 Fetoscopic laser 

• 43 Amnioreduction 

• Survival: Laser 61% AR 

51% 

− Both in same pregnancy 

54% 

− One or both survive 79% vs 

60% 

− Abnormal brain US 6% vs 

18% 

Vascular Equator 

Inter-twin Membrane


Selective Fetoscopic Laser Photocoagulation 

Fetal Survival At least 1 Survivor 

Hecher ‘99 61% 79% 

• Hecher ‘00 68% 81% 

• Quintero ‘00 61.3% 83% 

• Quintero ‘03 64.2% 83.2% 

• Huber ‘04 70% 83% 

• Huber ‘06 71.5% 83.5% 

• C Crombleholme ‘0 ‘07 77% % 91 91.7% 

% 

Vascular Equator 

Inter-twin Membrane

TTeam 

Pediatric surgeon 

Maternal Fetal 

Medicine 

Sonographer 

Cincinnati Mapping protocol 

1 st 

2 nd 

Identification of all vascular connection and 

recorded the findings. 

Confirmation of the vascular connection and 

laser photocoagulation. 

3rd No vascular connection was missed and 

Recorder no vessel had recanalized recanalized.

Positioning g and Set Up p


Anesthetic Options 

• Local with IV sedation 

• Posterior placentas only 

• Psychologically prepared 

• Calm and cooperative disposition 

• English speaking 

• Epidural with IV sedation 

• Standard approach 

• General anesthesthetic 

• Reserved for those with contraindications to regional 

or local anesthesia


• All have sequential compression stockings 

• All pressure points well padded 

• PPosterior t i placenta l t 

− Bump for uterine displacement 

− Side of recipient up 

• Anterior Placenta 

− Full lateral decubitus position 

− Flex the bed 

− Approach from side opposite donor with a window 

− Window between Pelvis 

and dth the costal t lmargin i

Fetoscopes Used in Laser Procedure 

• Storz fetoscopes 

• 3.3 mm 

• 1.8 mm semirigid lens 

• Must be hand curved to ~ 3- 3 degrees 

• Laser port 600µ endostat 

• Side port for Level I Rapid Infusor 

• Remote head scope requires req ires an 

Humanitarian exemption from the FDA 

• Can be inserted with or without a 

separate sheath 10 Fr Cook Introducer

Choice of Laser Used in Fetoscopic p 

Procedure 

• Neodymium-doped yttrium aluminium garnet 

− nd: YAG (Nd:Y (Nd:Y3Al Al5OO 12) ) crystal 

− Wavelength 1164 nm 

• Diode laser 

− semiconductor 

− Wavelength 980 nm 

nd:YAG 

Diode

Intraoperative Complications 

• Bleeding 

obscuring view 

• Level I 

amnioinfusion 

− Trocar insertion 

n=1 

− Chorionic plate n=1 

− Vessel laceration 

• 3 all controlled with 

Level I and Laser

Obscured Fetoscopic View 

• Mountainous topography 

• May be seen in prior AR 

• Amnioinfusion may flatten surface 

and facilitate visualization 

• Amnioinfusion may move donor twin 

or fl flatten tt the th iintertwin t t i membrane b onto t th the 

chorionic plate 

• Facilitate visualization of anterior 

placentas 

Level I Rapid 

Inf Infusion sion Device De ice


• Loss of amniotic cavity volume 

• Fracture of laser fiber 

• Loss of laser efficiency 

− Tangential angle 

− Th Through h iintertwin t t i 

membrane 

− Endostat problem 

• Debris 

• Fracture of the tip 

− Laser mal-alignment 

mal alignment 

600 µ Endostat fiber 

Amniotic litter


• Laser Microseptostomy? 

• Risk of monoamniotic 

gestation 

− Thought to be high with 

ultrasound guided 

needle 

• Risk of laser 

microseptostomy 

• 600 µ diameter width of 

laser fiber 

• MMonoamniotic i ti gestation t ti 

− Fetoscopy alone 1.3% 

− Microseptostomy in 

addition dd o 1.3% .3% 

− Does it help? 

− Restores fluid more 

quickly 

− Change in survival ?

Minimizing Vascular Shifts During 

Laser Photocoagulation 

• Sequential SFLP 

• AV AV, AA AA, VV 

• Requires detailed mapping 

• Improved p survival 

• Cincinnati mapping protocol 

• No laser of any vessel until the mapping is complete 

• Minimize laser time (usually ~ 5 minutes 

• Similar results

PPost-operative t ti Course C 

• Admit to FCC for 

tocodynomometric monitoring 

• Sequential compression 

stockings, foley 

• Clear liquid and advance 

• Tocolysis nifedipine with rate 

patient having Terbutaline 

• Ultrasound POD # 1 

• Discharge to from FCC in 

Cincinnati 

• Follow up fetal echocardiogram 

and ultrasound at POD #3

Quintero Staging of TTTS 

Stage I: Discordant amniotic fluid: DVP >8 cm & < 2 cm 

Stage II: Absent bladder in the donor 

Stage III: Doppler velocimetry changes in UA, UV, DV 

Stage IV: Hydrops 

St Stage V: V DDeath th of f one ffetus t

Acute Cardiac Changes in TTTS 

Quintero Staging 

I II III IV V 

Donor Recipient 

AEDF in UA Severe biventricular failure 

Reversal of flow in DV 

Severe TR/MR 

Reversal of flow in UA rec 

Early TTTS Endstage TTTS 

Staging is heavily weighted toward the donor 

Staging is heavily weighted toward the donor 

Recipient findings on in advance stage III and IV

10/10 

NIH TTTS Trial 

Logistic g Regression g Analysis y of Covariates 

Cardiovascular Profile Score (CVPS) 

Normal -1 point -2 points 

Hydrops None Ascites, pleural 

pericardial i di leffusion ff i 

skin edema 

Venous 

Dopplers Normal DV atrial reversals Venous pulsations 

CT Ratio < 0.35 > 0.35< 0.5 > 0.5 

Abnormal myo- Vent SF > 0.28 SF < 0.28 

cardial di l ffunction ti No AV regurg TR or semi lunar TR +dysfunction 

valve regurg any MR 

Abnormal Normal AEDF REDF 

Arterial 

Dopplers 

Cardiovascular Profile Score (CVPS). From Huhta, J Perinat Med 29:390-398, 2001

NIH Sponsored TTTS Trial 

LLogistic i i RRegression i AAnalysis l i of fC Covariates i on Survival S i l 

• Recipients 

− CCardiovascular di l Profile P fil Score* S * 

− Most predictive of poor recipient outcome 

• OR = 3.025/point 

• p = 0.05 

• Donors 

− Most predictive models of poor outcome 

− Increased Stage 

• OR = 0.446/stage p = 0.124 

− Earlier Gestational Age 

• OR = 1.052/day p = 0.097 

*Huhta et al 

J Perinat Med 29:390-398, 2001

Impact of Fetal Echocardiographic Findings 

on Recipient Survival 

•Cardiovascular Profile Score (CVPS) 

•62 consecutive recipient twins with TTTS 

• All patients treated by SFLP 

• All ll patients i had h d pre-operative i echo h evaluated l d 

• Blinded to outcome 

Michelfelder et al Ultrasound Obstet Gynecol 

30: 965-71, 2007 

Shah AD, et al:J Am Soc Echocardiogr 2008 

TR jet

SFLP 

100 

90 

80 

70 

60 

50 

40 

30 

20 

10 

0 

Recipient Survival Based on 

Pre-Operative CVPS 

p< 0.03 

p< 00.008 008 

10 of 10 9 of 10 8 of 10 

CVPS

Problem with the CVPS 

Normal -1 point -2 points 

Hydrops y p 

Venous 

None Ascites, ,ppleural 

pericardial effusion 

skin edema 

Dopplers Normal DV atrial reversals Venous pulsations 

CT Ratio < 0.35 > 0.35< 0.5 > 0.5 

Abnormal myo- y Vent SF > 0.28 SF < 0.28 

cardial function No AV regurg TR or semi lunar TR +dysfunction 

valve regurg any MR 

Abnormal 

Arterial 

Dopplers 

Normal AEDF REDF 

Cardiovascular Profile Score (CVPS). From Huhta, J Perinat Med 29:390-398, 2001 

Not sensitive enough

TTTS Cardiomyopathy 

•Fetal F lh hypertensive i cardiomyopathy 

di h 

− AV valve competence 

• Mild, moderate, severe TR/MR 

− HHypertrophy t h 

• End-diastolic LV, RV, Septum 

M di ld f ti 

− Myocardial dysfunction 

• Tei myocardial performance index


Cincinnati Modification of Quintero TTTS Staging 

Stage g I 

Stage II 

Stage III 

IIIA Mild cardiomyopathy 

IIIB Moderate cardiomyopathy 

IIIC S di h 

IIIC Severe cardiomyopathy 

Stage IV 

Stage V


Cincinnati Modification of Quintero TTTS Staging 

Stage g I 

Stage II 

Stage III 

IIIA RV MPI > 0.5 (Z+2) / LV MPI > 0.42 (Z+2) 

Mild ventricular hypertrophy (Z for RV/LV >2) 

Mild AV valve regurgitation 

IIIB RV MPI > 0.56 0 56 (Z+3) (Z 3) / LV MPI > 0.53 0 53 (Z+3) (Z 3) 

Moderate ventricular hypertrophy (Z for RV/LV >3) 

> Mod AV valve regurgitation 

IIIC RV/LV > Z+4Critical Doppler changes in RT 

(reversed DV, rUA) 

Severe TR/MR TR/MR, severe dysfunction 

Stage IV 

Stage V

Echocardiographic Features of TTTS by Stage 

Incidence of TR and MR 

100 

90 

80 

70 

60 

50 

40 

30 

20 

10 

0 

III IIIA IIIB IIIC IV 

Ci Cincinnati i ti Stage St 

Cincinnati Stage 

Tricuspid 

Regurgitation 

Mitral 

Regurgitation


Biventricular Hypertrophy 

Percent 

100 

90 

80 

70 

60 

50 

40 

30 

20 

10 

0 

Incidence of Biventricular 

Hypertrophy 

III IIIA IIIB IIIC IV 


Biventricular 

Hypertrophy

Tei Myocardial 

Performance Index 

Assessing Recipient TTTS Cardiomyopathy 

Tei C, et al. New index of combined systolic and diastolic myocardial performance: a 

simple and reproducible measure of cardiac function--a study in normals and dilated 

cardiomyopathy cardiomyopathy. J Cardiol 1995;26:357 1995;26:357-66 66 

Eidem BW, et al: Quantitative assessment of fetal ventricular function: establishing normal 

values of the myocardial performance index in the fetus. Echocardiography 2001;18:9-13 

Ichizuka K et al, The Tei index for evaluation of fetal myocardial performance in sick 

fetuses. Early Hum Dev 2005;81:273-9

Assessing Recipient TTTS Cardiomyopathy 

Tei Myocardial Performance Index 

*Identify Identify subtle alteration in ventricular function 

*Difficult to accurately measure 

*Inter-observer variability can be high 

*FCC echocardiographers h di h 10% variation i ti 

*Minimal change is >10% 

*Method to detect progression of TTTS during trial of AR 

*Underestimates dysfunction in moderate TR/MR 

*Can confirm post-treatment arrest of TTTS


Tei Index 

Myoccardial 

Peerformancce 

Index 

0.8 

0.7 

0.6 

0.5 

0.4 

0.3 

0.2 

01 0.1 

0 

III IIIA IIIB IIIC 


IV 

LV MPI 

RV MPI


TTTS cases since NIH Trial 

Closed May 2005 

570 TTTS patients 

Stage 

I 17.2% 

II 10 10.3% 3% 

III 11.5% 

IIIA 25.3% 

IIIB 18.4% 

IIIC 4.6% 

IV 69% 6.9% 

V 5.7% 

48.3% 

59 59.8% 8%

Distribution by Quintero Stage of TTTS 

Patients: The The Fetal Fetal Care Care Center of Cincinnati 

May 2005-January 2009 

570 patients 

300 

250 

200 

150 

100 

50 

0 

Quintero Stag 

0 I II III IV V 

Stag

Shift in Stage Distribution Comparing 

Quintero and Cincinnati Staging Systems 


570 patients p 

250 

200 

100 

Quintero vs Cinc 

150 Quintero 

50 

0 

0 I II III IIIa IIIb IIIc IV V 

Stag 

Cincinna

Percentage of Cases by Stage 

Upstaged by Echocardiographic Findings 


570 patients 0 

90 

80 

70250 

60 

50 

40 

30 

20 

10 

0 

200 

150 

100 

50 

0 

Percent Upstaged 

0 I II III III IIIa 

Stage 

IIIb III IIIc IV V 

Quintero Staging 

0 I II III IV V 

St Stage 

I II III

Echocardiographic Upstaging of Stage I 

Upstaging of Quintero's Stage I 

St Stage I 

39% 

70 patients 

Upstaged 

61% 

Stage I Stage IIIA Stage IIIB Stage IIIC 

Stage IIIA 

27% 

Stage IIIB 

14% 

Stage g IIIC 

20%

Echocardiographic Upstaging of Stage I 

Upstaging of Quintero Quintero'ss Stage II 

Stage II 

36% 

40 Patients 

Upstaged 

64% 

Stage IIIA 

16% 

Stage IIIB 

25% 

Stage IIIC 

23% 

Stage II Stage IIIA Stage IIIB Stage IIIC

Trial of Amnioreduction vs 


�175 patients stages I-IIIA 

�Normal �Normal fetal echocardiograms 

�Trial of AR with serial fetal echocardiographic assessmen 

�40% response to AR alone 

�60% progressed

Trial of Amnioreduction vs 


�AR responders survival 

�82.2% overall survival 

�90.7% 1 or both surviving 

�75% both surviving 

�AR non-responders-SFLP 

�79 �79.5% 5% overall survival 

�89.9% 1 or both surviving 

�70% both twin survive



�Stage Some IIIB, IIIC, IV 

�F �Fail iltil trial of fAR AR 

�Severe placental insufficiency

Acute Effect of SLFP on 

Tei e Myocardial yoc d Performance e o ce Index de 

•Pre and Post SFLP Echos 

•>10% improvement in Tei Index 

P 10% fall in Tei Index No Fall in Tei 

Habli et al 

Am J Obstet Gynecol Nov 2008

Overall Survival and Survival of 

One One or or Both Both Twins by Cincinnati Staging 


Cincinnati Stage

Post-Operative Complications in TTTS 

PPROM < 7 day : 5% 

PPROM > 7 day d : 22% 

Chorioamnionitis : 2.3% 

Transplacental trocar : 47% 

Yamamoto et al. 

Am J Obstet Gynecol. 2005 Sep;193(3 Pt 2):1110-6 

Iatrogenic monoamniocity 

Vascular accident 

Amniotic band syndrome 

Recurrent TTTS : 14% 

TAPS : 13% 

PPROM : 17% 

RRobyr b et al. l 

Am J Obstet Gynecol. 2006 Mar;194(3):796-803. 

?

AIMS 

• Incidence of complications after SFLP. 

• Distinguish early and late complications.

77 

Methods 

249 patient evaluated 

Sept. 2005 Feb. 2008 

Retrospective review 

14 6 SFLP (152) ( ) 

1152 2 

Amnioreduction alone (77) 

Radiofrecuency ablation (14) 

No treatment (6)

Methods 

Complications (maternal and fetal): 

• NNo complication li ti 

• Early complication: < 7 day after the 

procedure. 

• LLate t complication: li ti > >7 7 day d after ft the th 

procedure. 

• Early and late.

SFLP = 152 patients 

Results 

3 

(2%) 

149 

(98%) 

Twins 

Triplets

SFLP 

Result 

Serious complication or maternal deaths (0%) 

Overall Fetal survival (77%) 

Survival of one or both twins (88%) 

Recipient survival (83%) 

Donor survival (73%)

10% 

40% 

Complication 

29% 

21% 

No complication 

Early complication 

Late complication 

Early and late 

complication

10% 

40% 

Complication 

29% 

21% 

Early complication 

8% (12/152) PPROM. 

1.3% (2/152) Chorioamnionitis. 

11.3% 3% (2/152) Maternal 

peritoneal leak.

10% 

40% 

Complication 

29% 

Late complication 

• 17 17.8% 8% (27/152) PPROM 

• 8% (12/152) Abruption 

21% 

• 3.3% (5/152) ABS 

• 2% (3/152) TAPS 

• 2% (3/152) TTTS 

• 1.3% (2/152) Iatrogenic 

monoamniotic i i sac 

• 0.7% (1/152) Fetal hand necrosis 

due to vascular disruption p 

sequence

Impact of Complications on Survival 

No complication Early complication Late complication Both p value 

GA at procedure 21.0 + 2.5 21.3 + 2.6 21.1 + 2.4 22.2 + 2.5 N.S 

GA at delivery 

(weeks) 

Numbers DA-RV 

anastomoses 

Total number of 

anastomses 

coagulated 

31.4 + 4.1 28.3 + 5.7 30.1 + 3.1 29.8 + 3.8 p

Conclusions 

• SFLP Early, late and both early and late 

complications impact survival 

• Stringent placental mapping protocol Low rate of 

− TAPS (2%), 

− Recurrent/persistent TTTS (2%). (2%) 

• This information is important for counseling patients.

Pathophysiology of Recipient 

•Volume over load 

•Fetal systemic 

hypertension 

Cardiomyopathy 

− Estimates of 

ventricular ti l pressures 

based flow velocity of 

tricuspid regurgitation 

jt jet

Will Treating g TTTS Cardiomyopathy y p y 

Change Survival in Recipients? 

• Empiric treatment of TTTS cardiomyopathy 

with nifedipine 20 mg q 6 hours 

• Stages IIIA IIIA, IIIB IIIB, IIIC IIIC, IV 

• 20 mg q 6 hours 24-48 hours 

• 141 TTTS cases 

• 152 stage and GA matched controls 

• Significant reduction in tocolytics

• 

• 

Nifedipine 20 mg q 6 h 

Pre-op treatment 24-48 

100 

h 

90 

• 

• 

Case control study 

Matched for GA and 

80 

Ci Cincinnati i ti stage t 70 

• 

• 

Survival to birth 

Significant effect on 

60 

overall ll survival i l 50 

• Significant effect on 

recipient survival 

40 

• NNo effect ff ton DDonor 

survival 

30 

Effect of Nifedipine on Survival in 

TTTS Treated by SLFP 

20 

10 

0 

Nifedipine Control

Overall Survival and Survival of 

One or or Both Both Twins Twins in Stage IV IV TTTS 

TTTS 


Nifedipine effect?

Challenges g in TTTS 

• Define the etiology 

• Improving survival 

• Develop targeted medical therapies 

• Adj Adjunctive ti medical di l th therapy iin AR 

• Reducing morbidity 

• Defining cases to treat 

• Extend the length of gestation 

• Convincing the Obstetrical community

•Maternal-Fetal Medicine 

−Mounira Habli MD 

−William Polzin, MD 

−Kim Brady, MD 

−Jim VanHook, , MD 

−Ronald Jaekle,MD 

−David Lewis, MD 

•Fetal Echocardiography 

−Erik Michelfelder, MD 

−James |Cnota,MD | , 

−William Gottliebson, MD 

−Allison Divanovic,MD 

−Haleh Hadarian, MD 

•Urology/Nephrology 

−Pramod Reddy,MD 

−Robert Defore,MD 

−Elizabeth Jackson,MD 

•ENT 

−Ravi Ellarhu,MD 

−Paul Willging,MD 

•Neurosurgery 

−Karin Bierbrauer,MD 

−Francesco Mangano,MD 

•Cardiac Surgery 

−Pirooz Eghtesady,MD 

P M i MD 


•Pediatric Surgery 

•Timothy Crombleholme,MD 

•Foong-Yen Lim,MD 

•Sundeep Keswani MD 

•Anesthesiology 

–Ann Boat,MD 

–Mohammad Mahmoud,MD 

–Bonnie Hugus 

•Radiology 

•Connie Bitters 

•Linda Martin 

–Beth Kline-Fath, MD 

–Maria M i Calvo, C l MD 

–Eva Rubio, MD 

–Leann Linam, MD 

–Kyuran Choe, MD 

•Operating Room Nurses 

•Missy Ritter 

•Stacy Ruth 

•Tracy Heidrich 

•Latressa Latressa Ratner 

•Curtis Johnson 

•Neonatology 

− James Greenberg, MD 

− Tanya Cahill, MD 

− Kurt Schibler, MD 

− Beth Haberman, , MD 

− Paul Kingma, MD 

− Amy Nathan, MD 

•Nursing Director 

− Kelli Young 

•Coordinators 

− Gina Sharp, RN 

− Deb Voet, RN 

− Jenni Mason 

− Steve Imhoff 

− Judy Hostiuck 

•Genetics 

− Rob Hopkin,MD 

− Howard Saul,MD 

− Christine Spaeth, CGC 

− Diana Smith, CGC 

•Social Services 

− Erin Hartman, MSW 

•Administrative 

− Rachel Jones 

− Emmie Beyer 

Ch l S ll 

•Level III L&D Nurses 

• Good Samaritan Hospita 

•Nurse Midwife 

Karen McGirr 

−Peter Manning,MD − Cheryl Snell

Twin Twin Transfusion Syndrome: What are the ... - March of Dimes

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